written by C. McNamara Romanowski
As I crossed the expanse of desert and reservation between the train station and the city, I let my mind wander. I thought of Doug, 21 years old, headed back "home". Where was home? Now, home for me was a small apartment in a south-Phoenix suburb. I lived there with a yellow Labrador retriever named Troi, a shaggy, long-haired cat called Shorty, and a white-footed ferret who answered to Job (like the man in the Bible who lost everything so that God could prove a point to Satan).
Looking for change, less stress in my work environment, and broader career opportunities, I had come to Phoenix via voluntary job transfer. Being single, I had no one to depend upon except myself. At one time, I was married to Doug's father, but that hadn't worked out and I found myself raising him on my own. He was more than old enough now for me to make this move, I thought. My parents, both living, were only two hours away from where he attended school. They were elderly, and in relatively good health. My mother encouraged the move when I first took it under consideration.
My eyes drifted from the highway, across the desert. I watched a dust-devil pick up debris, sucking it up and around, up and around...small bits of inconsequential flotsam being guided sightlessly across the floor of the desert by a tiny tornado.
I navigated that desert road safely back to work, never realizing that I, too, was being sucked into something over which I had no control.
Weeks passed. I felt tired, achy. I noticed that sometimes I seemed to run out of breath where before I felt robust. I attributed it to the drier air of the desert.
With February, came news. My mother, who had been told that she had beaten breast cancer, was told that her cancer had returned. She would have to undergo further treatments...perhaps surgery.
It was frustrating being so far away. There was little I could do except offer kind words and prayers. I am an only child, so my parents had no other children to help them. They did find assistance through their church, which was an enormous comfort since I was unable to be there myself.
I moved through time as if I were not quite present. I was in Phoenix, but my heart was back in the cornfields of the Midwest. I talked with my manager about the possibility of doing my job from one of their Midwest locations. She told me this couldn't be considered until after I'd held the post for a year.
March. Spring break approached. I asked for time off work so that I could relax. Doug and I planned to do some marathon Internet gaming sessions, chasing down over-saturated monsters in imaginary forests and plains.
Leaving work that Friday, I felt happy. I always enjoyed playing online games...Besides, this would give me an opportunity to spend hours in conversation with my son. We are big talkers in my family. I stepped off the curb in the parking lot, and something in my ankle just gave way. I found myself unable to bear weight on my right leg.
In the Emergency Room, the doctor determined I had badly sprained my ankle, and put me in a partial cast. I was sent home on crutches with instructions that I was not to attempt bearing weight on that side for at least a week. After the week passed, I could remove the cast, and then would need to keep my foot elevated to avoid swelling and aid in healing.
I felt completely frustrated. How was I going to take Troi for walks? How was I going to get groceries, or even my mail?
I did manage...and I enjoyed my virtual, long-distance, exotic getaway of games with Doug.
Upon my return to work that next Monday, I should have felt rested, but the tired, achy feeling that had become so familiar was still with me. I did as I was supposed to, beginning to bear weight on my ankle, keeping my foot elevated, eating my veggies. It made no difference. I felt like I was in a whirlpool of swirling water, being sucked ever downward toward an unseen drain.
From time to time, over the years, I suffered tummy trouble that would leave me sitting on the porcelain throne, sweaty and quivering. It struck me once again about a month after my sprained ankle...with one glaring difference: There was blood in my stool. This was not just a tiny amount of blood, mind you. This was copious amounts of bright-red blood.
I called my doctor, with whom I had just become established because of my recent relocation. There were no appointments available. The receptionist recommended I visit a walk-in clinic. I found one near where I lived and worked. After waiting for what seemed like an hour but was probably more like two, the doctor listened to my stomach with a stethoscope and said I had the stomach flu. I told him about the blood, and he didn't even blink. Anti-biotics were prescribed, and I was sent home.
A week later, April 29, I went back to work. I felt pretty guilty about missing another week of work so soon after my spring-break holiday. I was still weak and "under the weather", but I wanted my boss and co-workers to know that I cared about my job, and I didn't want to let them down. A few people remarked that I didn't look like I felt very good.
Around mid-morning, I noticed tiny, red dots appearing on the inside of my wrists. They were very small, about the size of a pore. 'Perhaps an allergy,' I thought. It wouldn't be the first time. But there was no itching, and it didn't look like any rash I'd had before.
Again, I called my doctor. This time, the receptionist told me that I could be seen that afternoon, at 3:15. I arranged to leave the office a little early, and made it to the doctor's office in time for my appointment. I remember being very, very tired. I didn't even want to sit up when the doctor entered the exam room.
We began to discuss what had been going on with me. She looked at my speckled wrists, and said, "I'd like you to go and get some blood drawn, and I want you to have a chest x-ray."
Looking at her watch, she realized how late in the day on Friday it was. "Actually," she said, "I want you to go to the Emergency Room. This can't wait over the weekend. Go there now, and tell them you need a chest x-ray and a CBC."
The doctor repeated these instructions, and sent me on my way.
I went home, took Troi out for her afternoon constitutional, then headed to the hospital Emergency Room. This was the same ER I had visited just a month before when I sprained my ankle.
The intake aid didn't seem to feel I was in the right place. "Aren't you supposed to go to a lab?" she asked.
I explained that my doctor didn't want to wait for the results and that I had been instructed to go to the ER. Blood pressure was taken, temperature noted, and I was asked to have a seat in the waiting area.
I generally have low blood pressure, around 90/60. At this point, I should have known something was wrong because the aid said my BP was normal (i.e. 120/80). I have often had the suspicion that aids who take my BP sometimes just say it's normal because it's difficult to get a reading on a person with a low, quiet blood pressure. You will see why this is significant later...
Several hours passed. I entertained myself with a cellphone game until my battery gave out. After that, I just watched the people come and go, realizing that I had been shuffled to the back of the line because it didn't seem like my problem was so acute that I couldn't wait.
Around 10 pm I was finally ushered back into the area of the building where patients were being seen. The examining rooms were full, so I was placed on a gurney in the hallway.
A large, jolly-looking physician's assistant came to talk with me. He looked at my wrists, and then asked if I had the speckles on my feet. I didn't know, so I removed my shoes.
My feet and ankles were covered in tiny, red dots. I asked the guy what he thought they were. He smiled and said we were going to find out.
I was taken for a chest x-ray. A woman drew my blood. Time passed. Some time after I was returned to my perch on the gurney, I watched a family down the hall react as they were informed of the death of a loved one. It made me feel like a voyeur, despising whoever made the decision to have me placed in the hallway.
Finally near midnight, a very serious young doctor came to me and asked, "How long have you been anemic?"
"I was first told I had anemia when I was a child, around age 8," I said.
"Has anyone ever talked to you about your platelets?" he asked.
"Once, when I was 28, some pre-op blood work showed an abnormality. When I asked about it, the nurse told me not to worry about it." I started to feel a little panicky. Platelets?
"Well, your hemoglobin is 8," he said. The lowest I had known it to be prior to this was 10. "You're going to need a transfusion."
"Your platelets are 6."
I know I had a look of confusion on my face. I didn't know what a good platelet count was. The doctor took my cue.
"Your count should be 100 or higher."
I knew enough to know that platelets are what clot your blood, and if you don't have any you bleed to death.
"We're going to need to run some tests. I want you on bed-rest, except to go to the bathroom. If you're very careful, you can get up to use the bathroom. A slight bump could be catastrophic."
I tried to bargain with him so that I could go home. I didn't want to stay in this place. I didn't want to sleep in their bed and eat their food. I didn't want needles in me and people prodding me. I didn't want any of this. I felt myself screaming...on the inside.
He would have none of it. I was going to stay.
A couple of hours later I was settled into my room in the hospital. The nurse announced my blood pressure was 60/50. She said this was because of anemia. (Remember that 120/80 from earlier in the evening?) An IV was installed, blood was ordered, aspirin given. I guess they dose you with aspirin before a transfusion because some people receiving blood develop certain issues.
Though my time in Phoenix had been short, I had become well-acquainted with one of my neighbors, Connie, who was also from the Midwest. I telephoned her from my bedside, and asked her to take care of my animals during my absence. She quickly agreed, and assured me that they were in good hands.
A nurse sat with me throughout the entire transfusion of two units of whole blood. He said that he was there to monitor my body's reaction to the introduction of fluids and foreign substances.
This fella was a tall, muscular, Native American man with long, black hair. I don't remember his name. We talked a bit. I remember him telling me that he was a "floater" who worked the Phoenix area for three weeks, then returned to his home on the reservation for a week. Rinse, repeat.
Could this really be happening to me?
The little dots on my wrists and feet were called "petechiae" (pronounced puh-teek-ee-yuh). They were a definitive sign that I had a low platelet count. Think of them as tiny hemorrhages just under the surface of the skin.
At least that mystery was solved. But what was wrong with me?
In the morning, a doctor came in to see how I was doing. He said that I was going to have multiple workups to determine the cause of my condition. When I asked him what this could be, he was careful in his wording and broke it down for me.
I heard, "...leukemia...lymphoma..."
After days of poking, prodding, and general discomfort, all kinds of tests...even a bone marrow biopsy...I was introduced to my new hematologist, Dr. Mikhail Shtivelband. It was May 3, 2005. In just a few days I would turn 44.
Dr. Shtivelband was a slender man of middle years, gentle in spirit, dark of hair and eye. He spoke with a distinctly Russian accent.
"Your direct antiglobulin Coombs test is positive. You have idiopathic thrombocytopenic purpura. This is idiopathic because we do not know its source. It is unexplained. This is responsible for your drop in platelet count." He continued, "You also have autoimmune hemolytic anemia. These two things usually occur separately. but can occur together. When they occur together, we call them Evans Syndrome."
Dr. Shtivelband explained to me that most of the time Evans Syndrome presented in childhood. He said it is usually accompanied by another autoimmune condition, such as rheumatoid arthritis. He explained what my first line of treatment would be: IV steroids and IVIg. I had absolutely no idea what IVIg was.
"This is a blood derivative that will essentially coat your blood cells in order to fool your body so that the cells are not destroyed. It is only temporary."
More blood products. Great.
"IVIg is difficult to obtain in large quantities, but we should be able to have it here to administer by tomorrow afternoon," he continued. "We will also administer IV steroids because it has the effect of shutting down the immune response in the body."
Well, that doesn't sound so bad, I thought. Not so bad...
As it turns out, IVIg is the acronym for Intravenous Immunoglobulin, derived from human blood donations. It takes a portion of the blood from over 1,000 donors to make up just one bottle of IVIg. Its effect lasts between two weeks and three months, and the cost is well over $10,000 US. At the time of this writing, I found data that showed the cost of IVIg to be on the rise...expensive little bottle of life.
Steroids were started right away. IVIg was added the following afternoon. The nurse told me that we were lucky to get it.
I spoke on the phone with my son, my parents, my boyfriend, William. They were all so far away. I was spending my time in the desert...40 days and 40 nights of exile in the heat, baking in the white-hot sun of self-examination.
"It has a name," I told William. "It's called Evans Syndrome."
This was only the beginning.
Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.